Definition of Calcification, nonarteriosclerotic cerebral
Calcification, nonarteriosclerotic cerebral definition - medical term This condition described
in 1930 by T. Fahr (and therefore called Fahr syndrome) is a genetic (inherited)
neurological disorder characterized by abnormal deposits of calcium in certain of
areas of the brain (including the basal ganglia and the cerebral cortex).
S ymptoms may include motor function deterioration, dementia, mental retardation,
spastic paralysis, dysarthria (poorly articulated speech), spasticity (stiffness
of the limbs), ocular (eye) problems, and athetosis (involuntary, writhing movements).
Features of Parkinson's disease such as tremors, rigidity (resistance to imposed
movement), a mask-like facial appearance, shuffling gait, and a "pill-rolling" motion
of the fingers may also occur in individuals with Fahr's syndrome. Other symptoms
may include dystonia (disordered muscle tone), chorea (involuntary, rapid, jerky
movements), and seizures. Onset of the disorder may occur at any time from childhood
to adulthood.
Fahr syndrome thus involves abnormalities of the neurologic system (cerebral
calcification, dementia, spastic paraplegia, athetosis), skull (microcephaly, i.e.
an abnormally small head), eyes (glaucoma, optic nerve atrophy, retinitis pigmentosa),
and, we would add, a significant hormone problem, namely hypoparathyroidism (the
parathyroid gland regulates calcium).
The disease is inherited as an autosomal recessive trait in which both parents
carry a Fahr gene and each of their children (boys and girls alike) stands a 1 on
4 (25%) risk of receiving both Fahr genes and therefore having this dread disease.
There is no cure for Fahr's syndrome, nor is there a standard course of treatment.
Treatment is directed toward minimizing symptoms.
The prognosis (outlook) for individuals with Fahr's syndrome is poor. Progressive
neurological deterioration generally results in disability and death.
Alternative names for this syndrome include striopallidodentate calcinosis, SPD
calcinosis, and cerebrovascular ferrocalcinosis.
|
|
